Dominantly inherited cystoid macular edema

Abstract

An apparently autosomal-dominant macular dystrophy occurred in three pedigrees with the presenting signs of typical cystoid macular edema due to leaking perimacular capillaries. Other striking features were retinal capillary leakage all over the posterior pole of the eye, whitish punctate deposits in the vitreous body, a normal electroretinogram, a subnormal electro-oculogram, and moderate to high hyperopia. In later or more advanced stages the macula developed a central zone of "beaten bronze" atrophy. Strabismus occurred frequently.